Intractable gastrointestinal bleeding from angiodysplasia in a patient with Bernard–Soulier syndrome

Ouakaa-Kchaou, Asma; Gargouri, Dalila; Elloumi, Héla; Kharrat, Jamel; Ghorbel, Abdeljabbar

doi:10.1007/s00277-010-1116-9

Cited by 3 publications

(1 citation statement)

References 9 publications

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“…Common symptoms include epistaxis, ecchymoses, menorrhagia, gingival and cutaneous bleeding, gastrointestinal bleeding and bleeding after trauma or surgery [3,5]. Angiodyplasia in patients with BSS resulting in severe gastrointestinal bleeding has been described [6]. Often, bleeding and bruising are more significant than expected from the degree of thrombocytopenia [3,7].…”

Section: Clinical Presentationmentioning

confidence: 99%

Inherited platelet disorders: a clinical approach to diagnosis and management

Cox

Price

Kahr

2011

Expert Review of Hematology

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Inherited platelet disorders encompass a heterogeneous group of bleeding disorders where a variety of molecular defects can affect platelet number, function or both. The defects involve deficiencies or dysfunction of platelet receptors, signaling pathways, cytoskeletal proteins, granule contents and abnormalities in procoagulant activity. These disorders can be difficult to distinguish clinically as they present with the common symptom of mucocutaneous bleeding. Inherited thrombocytopenia needs to be considered in all patients suspected of having primary immune thrombocytopenia, where platelets may also have functional defects. After a careful history and physical examination, initial investigations include a complete blood count with a peripheral smear, followed by appropriate specific investigations that often require specialized referral centers. This article is a summary of the current data on clinical presentation, pathogenesis, diagnosis and management of inherited platelet disorders.

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Section: Clinical Presentationmentioning

confidence: 99%

Inherited platelet disorders: a clinical approach to diagnosis and management

Cox

Price

Kahr

2011

Expert Review of Hematology

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Recurrent jejunal bleeding due to angiodysplasia in a Bernard–Soulier patient

Otrock

Degheili²,

Sibai³

et al. 2013

Blood Coagulation & Fibrinolysis

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Angiodysplasia of the gastrointestinal tract consists of ectasia of the submucosal vessels of the bowels and may lead to acute or chronic bleeding. The presence of a coagulopathy will increase the frequency and the severity of gastrointestinal bleeding, whether spontaneously or medically. In literature, few cases of Bernard-Soulier syndrome associated with gastrointestinal angiodysplasia have been reported. We hereby present a female patient known to have Bernard-Soulier syndrome presenting with persistent bleeding due to jejunal angiodysplasia, which, to our knowledge, is the eighth reported case in the medical literature. The patient responded to tranexamic acid (antifibrinolytic agent) with gradual reduction in required transfusions until cessation of bleeding.

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